Gliomatosis cerebri

Orpha code: 251582OMIM code:

Definition

A rare glial tumor characterized by extensive infiltration of the brain, often extending to infratentorial structures and even the spinal cord. The tumor corresponds to WHO grade III and is composed of elongated glial cells typically resembling astrocytes. Cases in which the predominant cell type is oligodendroglial have also been described. Some tumors develop a circumscribed neoplastic mass in addition to the diffuse lesion, usually showing features of high-grade glioma. Clinical symptoms include dementia, headache, seizures, signs of increased intracranial pressure, and a variety of neurological deficits. Prognosis is generally poor.

Disease data
Classification

Disease

ORPHA code
251582
OMIM code
-
ICD10 code
C71.0
ICD11 code
2A00.0Y

No additional description.

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